Background: Infective endocarditis (IE) in children may be difficult to diagnose specially if there is no underlying heart lesion. Aim: to review the incidence of infective endocarditis (IE) as a complication of sepsis in pediatric patients with normal heart, the presenting signs and symptoms, as well as bacteriological etiologies in infants and children with IE, focusing on the risk factors. Patients and Methods: Fifty patients were selected, they were suspected clinically as septicemic and proved by history, clinical examination, laboratory investigations as complete blood picture, acute phase reactants as erythrocyte sedimentation rate, C-reactive protein, urine analysis & culture and repeated blood cultures. Localizing signs of infection as pneumonia, osteomyelitis, skin abscess, meningitis, urinary tract infection may be a presenting sign. Echocardiogram with color Doppler was performed for all septic patients for detection of valvular vegetations, its sequelae and other complications. Results: 50 studied cases; 35 neonates, 7 infants and 8 children, their age ranged from 1 day to 14 years, with a body weight ranged from 1.9Kg to 40Kg. There was male predominance; 1.7/1 in neonatal period, 1.5/1 in infancy and childhood. Enterobacter is the commonest organism, isolated from 13 out of 50 cases (26%). IE was a localizing cardiac complication in 4 out of 50 cases with sepsis (8%); all cases were children with age ranged from 3 to 13 years. All children with IE were presented with fever, dyspnea, tachycardia, with appearance of new pansystolic murmur at tricuspid area. Staphylococcus aureus was the causative organism in 1/50 (25%) of IE patients, enterobacter species in 1/50 (25%), and 50% were culture-negative endocarditis. All affected cases had right-sided (tricuspid valve) endocarditis. The factors predisposing to IE were not well understood, bacteremia arising from infected bones, joints, skin lesions and obstructed ventriculoperitoneal shunt were the presumed sources of valvular involvement in these children. Conclusion: this study should alert the clinician to carry out an exhaustive work in critically ill children with bacteremia for exclusion of endocarditis in this risky group of patients with normal heart structure. The diagnosis of infective endocarditis can be made in the patients with bacteremia and sepsis with compatible clinical symptoms and signs and echocardiographic findings of vegetations. The diagnosis should be suspected in any septic child with a newly discovered cardiac murmur and prolonged fever.